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   Table of Contents - Current issue
November 2017
Volume 4 | Issue 3 (Supplement)
Page Nos. 1-206

Online since Tuesday, November 21, 2017

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The power of determination Highly accessed article p. 1
Karoon Agrawal
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From president's desk: Special issue for cleft congress 2017 p. 2
Jyotsna Murthy
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Report of the scientific chair: Cleft 2017 p. 3
Krishnamurthy Bonanthaya
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International Confederation for Cleft Lip and Palate and Related Craniofacial Anomalies 2017 Task Force Summary Reports p. 4
Jeffrey L Marsh
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Quality of care in Robin sequence p. 10
Corstiaan C Breugem, Maartje Haasnoot
Robin sequence is a heterogenous phenomenon with high morbidity and even mortality. The sequence is often associated with other anomalies, however it is of utmost importance to adequatly treat the upper airway obstruction. In a time where value of health care is judged by objective outcome measures, Robin sequence is in need to have these outcome measures better defined. This manuscript will summarize some important aspects that could help us to determine objective outcomes to measure treatment success.
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Facial deformity correction: A journey through small towns and rural India p. 16
Sailesh Kumar Mukul, Abhishek Singh, Sumit Singh, Priyankar Singh, Amit Kumar
Introduction: Craniofacial anomalies constitute a large fraction of facial deformity cases. The aim of this study is to develop a road map to extend the services for facial deformity correction in small towns and rural parts of the country. The objectives were to assess the existing services for facial deformity correction as a pilot project and to conduct strength, weakness, opportunities, threats analysis of existing care providers for facial deformity correction in Bihar. Methods: A questionnaire was generated titled “Survey on Distribution, Management, Difficulties Encountered and suggestions to improve services to manage the facial deformity cases in a conventional small surgical setup in Bihar” and was distributed among existing care providers for the facial deformity patient. Results: Less than 30% of care providers provide services at small towns and villages. Majority of care providers are centered at the district level. Discussion: The paucity of care providers for corrections of facial deformities needs to increase from mere 30%.This relates to desired increase in capacity by two folds to clear the backlog in a decade in India. Conclusion: At present, no national level policy to address the facial deformities which require to be formulated through established national and international societies and organizations.
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A new classification approach: Center of integral care of cleft lip palate “SUMA” center (México) p. 20
Jose Maya Behar, Daniel De Luna Gallardo, Rodrigo Morales De La Cerda, Silverio Tovar Zamudio
The cleft lip palate (CLP) is characterized by multiple phenotypic presentations in relation to the degree of severity and complexity that may involve its embryology. These characteristics largely determine the approach and therapeutic plan. There have been many descriptive, diagrammatic, and coding systems throughout history that have tried to define and unify the malformation. However, there are only a few that have stood the test of time. The objective of the present study is to propose a new classification of CLP (“SUMA”) by an observational study implemented in 410 patients. We use the criteria proposed by Hakins (1961) for an “Ideal” classification to evaluate our system. The 406 patients (99%) fulfilled the criteria in their totality being able to be classified adequately. Proving that the “SUMA” system corresponds to a unique, descriptive, reproducible, clear, and specific tool that promises to unify the classification in the CLP.
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Comparison of craniodentofacial morphology of children with and without unilateral cleft lip and palate p. 25
Servet Dogan, Sultan Olmez, Ege Dogan
Background: This retrospective case-control study was done to investigate the cranio-dento-facial morphology of Turkish children with unilateral complete cleft lip and palate from Ege University and compare them with a normal group to highlight the effect of surgical correction on craniofacial development. Methods: 60 patients with unilateral complete cleft lip and palate were compared with 55 control children without cleft at mean ages of 13 and 15 years. The cleft lip was operated with modified Millard technique at 3 months, cleft palate was operated with von Langenbeck technique at 12 months. The patients were not given any orthopaedic or orthodontic treatment also. Angular and linear measurements of cranio-dento-facial complex were measured by using Dolphin Imaging 11.5 software programme, and the resulting data were evaluated statistically. Results: The children with unilateral complete cleft lip and palate had considerable morphological deviations when compared with the matched children without clefts. The most striking findings in the unilateral complete cleft lip and palate group: Maxillary and mandibular complex and nasal projections were retruded when compared with normal (P<0.001). Also, nasal airway dimensions were higher in control group (P<0.001). Soft tissue structure, especially upper lip thickness, upper and lower lip lengths were decreased (P<0.001, P<0.001, P<0.05, respectively) in cleft group. Maxillar anterior height was decreased (P<0.001). Besides, nasolabial angle and upper incisor inclination according to the maxillary occlusal plane (P<0.001) were increased while overjet (Mx1-Md1) were decreased (P<0.001). Conclusion: The surgical technique and the number of operations seem to be the most effective factors for maxillary retrusion in patients with unilateral complete cleft lip and palate.
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Evaluation of MSX1 gene as the common candidate gene of nonsyndromic congenital hypodontia and cleft lip and palate p. 31
Ayse Tuba Altug, Asli Senol, Ozlem Nasibe Ozkepir, Haldun Dogan, Serdar Ceylaner, Erhan Ozdiler
Introduction and Aim: Cleft lip and palate (CLP) is complex craniofacial deformity in which both the environmental and genetic factors play a role. Congenital hypodontia is the absence of permanent teeth. As congenitally missing maxillary lateral incisors (CMML) and CLP both occur at the suture between premaxilla and maxillary posterior segments, this finding has directed us to investigate if both anomalies could be affected by the same genetic factors. Therefore, the aim of our study is to investigate if there is a common genetic pattern between the occurrence of CLP and congenitally CMMLs. Subjects and Methods: Muscle Segment Homeobox-1 (MSX1) is one of the common candidate genes of hypodontia and CLP. In this study, the role of MSX1 for CLP and CMML was evaluated. The CLP and CMML groups were consisted of 51 and 48 participants, respectively. 3cc blood samples with EDTA were collected and genomic deoxyribonucleic acids were isolated. To screen for putative mutations, two exons of MSX1 gene as well as their exon–intron boundaries were amplified by the PCR and analyzed with Sanger sequencing method. Results: In both groups, the same SNP (c. *6C>T, rs 8670) which is localized in 3'untranslated region of MSX1 gene was detected. Minor allele frequency, heterozygosity, and Chi-square test for Hardy–Weinberg equilibrium at c. *6C>T variation were computed. The expected wild-type, heterozygous, and homozygous allele frequencies of c. *6C>T variation were % 65.61, % 30.78, and % 3.61, respectively. However, the frequencies were %47.9, %45.8, and %6.3 in CMML group and %80.4, %11.8, and % 7.8 in CLP group. These frequencies were diverted from normal for both groups, and the differences between the groups were statistically significant P < 000.1 (Chi-square test). Conclusion: The existence of common polymorphisms and diversions from the normal population in the 3'untranslated region of the MSX1 gene is supporting the hypothesis of a possible relationship between CLP and CMML incisors.
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Lip prints as a genetic marker in inheritance of cleft lip and palate: A case–control study p. 38
Abhilasha Yadav, Rahul Gaikwad, Anuj Jain, Suhas Jajoo
Aim: The aim of the study was to study the various pattern types of lip prints in parents of cleft lip and palate (CLP) siblings to detect if any specific pattern can be considered as a genetic marker. Patients and Methods: The study included 100 participants (study group parents with children having CLP, fifty fathers and fifty mothers) and fifty participants (control group parents having children without CLP, 25 fathers and 25 mothers. The lip prints of the participants were obtained using the cellophane method and analyzed using Suzuki and Tsuchihashi classification of lip prints. The data were subjected to Chi-square test. Results: On the basis of the logistic regression, prediction of 27% of abnormal pattern in more than one-quadrant of lip can be considered as genetic marker for cleft lip (palate) deformity. Discussion: In our study, abnormal pattern Type V had been found which was considered as genetic marker for transmission of cleft lip (palate) deformity and a predominant pattern of Type II was found which was same in lines of An Egyptian study. Conclusion: A highly significant correlation was observed in lip patterns in parents with cleft anomalies.
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Controversy and consensus for primary repair of cleft lip nose deformity p. 43
Raj Kumar Manas, Shamendra Anand Sahu
Introduction: Correction of Cleft lip nose deformity is a difficult challenge for cleft surgeons. Though, Primary repair of cleft lip nose deformity has been accepted worldwide, there are many variations amongst surgeons involved in cleft care. To demystifying the controversy of primary correction of cleft lip nose repair, we have done an internet based survey to define consensus among surgeons. Methodology: A 15 items questionnaire was framed and sent to the all plastic surgeons of India as well as oral & maxillofacial surgeons, Head & neck, ENT & Pediatric surgeons of the country involved in cleft lip surgery. The questionnaire was categorised in the form of repairing the nose deformity with cleft lip, approach to address such deformity, secondary complications & residual deformity present and satisfactory outcome in order to make a common consensus among surgeons involved in cleft surgery. Results: Of total respondents, 91.8% were plastic surgeons and 9.3 % Oral & maxillofacial surgeons. Almost all of them(100%) felt that nose deformity is a part of cleft lip, but only 81.3% agreed for nose deformity correction at the same time of cleft lip repair. Those who performed nose correction were about 58.1%, who always addressed nose deformity whereas 38.7% performed it sometimes.68.8 % address mostly flaring of ala whereas 52.1% address depressed nasal dome also while 24% address all the deformities. The maximum group, 45.3% prefer semi-open approach; whereas almost equal group 42.1% feel closed approach is sufficient enough to address all deformities. As per age of primary correction of nose deformity, 43% do it at the age of 3-4 months along with cleft lip repair whereas 33 % wait for 6 months. Regarding extent of dissection, 31.2% believe in minimum dissection, whereas for 62.7% it varies according to deformity. 89.7% feel primary correction reduces the deformities and 54.1% people feel only 25% patients may require revision before 7 years of age. However 59.6% prefer to revise the nose correction secondarily at adolescent age. About 29.3% surgeons were satisfied with their result in almost 50-75% of their patients and equal group in their 25-50% of patients' .The main complication they encounter was residual nose deformity in about 75.8%. About 87.8% believe there is no maxillary growth disturbances following nose repair and 87.52% are in favour of doing nose correction at the time of cleft lip repair. Conclusion: To conclude, nose deformity is a part of cleft lip and should be repaired at the time of cleft lip repair. The optimum age of repair is same as for cleft lip. The approach may be “semi-open” to “closed” depending on the surgeon's expertise and experiences and extent of dissection may vary according to deformities. However, a small group of patients may require secondary correction, but it is evident that primary nasal correction reduces the deformities and it should be an integral part of cleft lip repair.
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Reliability and validity of tomographic anthropometric parameters in patients with cleft lip and palate p. 50
Tatiana Izchel Castillo Torres, Marcia Rosario Perez Dosal, Ariana Cruz Sandoval, Norma Mónica Dominguez Martinez
Background: The main stigma of cleft lip and palate (CLP) is the nasal asymmetry, anthropometry helps to evaluate results, the computerized tomography (TC) assesses the maxillary conditions, and the three-dimensional (3D) reconstruction can aide to evaluate soft tissues. Repeatability and validity of tomographic anthropometric parameters of nasal symmetry have not been estimated before. Aims: The aim of the study was to estimate the repeatability and validity of tomographic nasal symmetry anthropometric parameters in patients with CLP. Settings and Design: This study was a cross-sectional study. The sample size was 63 measurements in patients with CLP 9–18 years. Variables such as sex, age, clinics and tomographic anthropometric measurements, Sn-C, Sn-Prn, A, Gsup-Gbase, Ac-Prn, C, D, Nt-Nm/Nb-Nl, La med-La lat, Sa inf-Sa sup, Sn-Al, B, Sn-Ac, Sn-Sbal were used. Subjects and Methods: Two measurements of reconstructed anthropometric tomographic 3D were evaluated twice to assess repeatability. Validity of tomographic was calculated comparing with clinical anthropometric measurements (gold standard). Statistical Analysis: Intraclass correlation coefficient was used to assess the repeatability and the individual differences for criterion validity. Results: Fifty-one subjects were included, mean age was 12.82 ± 2, and 64.71% were males. Sn-C, Sn-Prn, A, Gsup-Gbase, C, D, Nt-Nm/Nb-Nl, La med–La lat, Sa sup–Sa inf, Sn-Al, B, Sn–Ac, and Sn-Sbal had a repeatability CCI above 0.70. For validity, Sn-Prn, Prn-Ac, D, Nt-Nm/Nb-Nl, La med-La lat, Sa sup-Sa inf, Sn-Al, B, Ac-Sn, and Sn-Sbal tomographic measurements showed a mean difference ≤1 mm in comparison with clinical anthropometric measurements. Conclusions: Nine tomographic measures were closer to clinical measurements. The tomographic measures are a good way to evaluate nose symmetry in patients with CLP.
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Extracorporeal septoplasty: Role in correction of unilateral cleft nose p. 61
BS Jayanth, Abhitosh Debata
Purpose: To evaluate the functional , aesthetic outcomes, and complications associated with extracorporeal septoplasty (ECS) while correcting moderate to severely deviated nasal septum in unilateral cleft lip and palate (CLP) patients. Materials and Method: Fifteen healthy adolescent and adult unilateral cleft lip and palate patients reporting for cleft rhinoplasty procedure to our department and consenting for the research protocol were taken up for study. Result: Mean duration of ECS was 40 minutes. Adequate cartilage graft from excess septum was available for aesthetic and functional requirements in 100% cases. Post-operatively, overall pain scale scores ranged from moderate to no pain. Nasal Obstruction Symptom Evaluation (NOSE) scale scores were significantly low post-operatively in comparison to baseline values (pre – 46.67; 1st Post-operative month – 21.33, 3rdPost-operative month – 16, 6th Post-operative month - 15). The only Intra-operative complicationsnoted were mucosal tear (40%), haemorrhage (6.7%)with absolutely no early or delayed post-operative complications. According to modified-Asher – Mcdade index, nasal form, nasal profile and deviation of nose hadfair to good appearance throughout the case series. Conclusion: ECS is safe and versatile procedure to amply address the septal deformity in all dimensions and offers adequate septal cartilage from the excess septum in cleft nose that can be used as sole graft for all reconstructive requirements in rhinoplasty with improved aesthetics and no additional intra-operative time, cost and complications
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Evolution of a safe and effective management protocol for velopharyngeal incompetence: Seeking good speech without airway compromise p. 68
John H Grant, Meghan Amerson, Laura Sconyers, Ahmed Elsherbiny
Objective: To document an evidence based evolution of VPI management over an 18 year period with the goal of optimizing speech while minimizing complications and need for re-operation. Design: An institutional review board approved, retrospective 18-year single surgeon, single-center series of 370 patients undergoing surgery for velopharyngeal incompetence (VPI). Methods: A review of our database between the years of 1997 and 2015 identified 370 patients who underwent surgical procedures for VPI. Records were reviewed for types of procedures performed each year. Procedures were evaluated for safety and efficacy as regards to speech outcome. The study population was divided into an earlier period (1997–2009) and a later period (2010–2015). Results: We demonstrated a reduction in the use of airway obstructive procedures such as sphincter pharyngoplasty from 25.2% of VPI surgeries in the early period to 10.4% in the late period. There has been a progressive shift toward more physiologic procedures such as re-repair with intravelar veloplasty (IVVP). The extent of IVVP has become more aggressive over the time period of the study. In our re-repair population, 89.7% of patients had improvement of the speech scores with 64.5% of all the re-repair patients achieving normal speech postoperatively. Autologous fat augmentation of the palate was introduced in the second period and represents about half of the procedures in the later period. Focused autologous fat augmentation of the palate resulted in statistically significant improvement in speech in 75.7% of cases. Conclusion: This study demonstrates an evidence-based evolution in management, shifting away from potentially airway obstructive procedures and toward a more physiologic approach to velopharyngeal competence. We outline the steps taken to reach the author's current approach to VPI management based on periodic outcome audits, specific anatomic findings, and goal-directed surgical interventions. With correct diagnosis and patient selection, VPI can be safely eliminated in over 70% of patients with a single procedure. We believe that this anatomic and physiologic approach improves safety while minimizing risks of airway obstruction.
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Implant rehabilitation in cleft patients: A retrospective study in our center p. 73
José-Luis López-Cedrún Cembranos, David Neagu, Ramón Luaces Rey, Maria Josefa Pombo Castro, Alicia González Mourelle, Francisco Gálvez Prieto
Introduction: One of the objectives of alveoloplasty and alveolar bone grafting is to create a stable and continuous maxillary arch that allows teeth eruption through the bone graft. The dental rehabilitation by means of implants is a good technique for prosthodontic rehabilitation of edentulous space in cleft patients. The aim of this study is to review our series of patients with clefts rehabilitated with dental implants. Materials and Methods: A retrospective study of 25 patients treated by means a dental rehabilitation based in implants has been realized. We have evaluated the type of alveoloplasty, the age of the patient at the time of implant insertion, the success of the procedure and the need of additional techniques before or at the time of the procedure. Results: Before the insertion of implants, 11 patients had received a secondary alveoloplasty, 10 patients a late alveoloplasty, and four patients did not received an alveoloplasty procedure. A total of 47 implants have been inserted. The mean follow-up ranged between 6 and 153 months. No implants were lost in this period. The mean age of insertion of implants varies regarding the type of alveoloplasty. Four patients needed a bone block graft before the implant insertion, one was treated with a sinus lift, and nine patients received a supplement of bone at the time of insertion of the implant. Conclusions: Dental rehabilitation with implants is a safe procedure to close the edentulous gap in cleft patients.
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The use of tongue flaps in primary cleft palate repair p. 78
Navin Vig, Atheer Ujam, Hamza Elburi
The tongue flap, for the reconstruction of oral defects, was first described over one hundred years ago. They have since been used to manage a variety of defects and have proved to be reliable and predictable. We report our preliminary experience with the use of the anterior-based pedicle tongue flap for primary repair of wide palatal clefts in Libyan infants. The repair was associated with good structural closure of the defect and was free of any major complications. Given the current limitations in Libya, we have used this method for wide palatal cleft defects in the absence of standard support from a multi-disciplinary team. Our initial results look promising.
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Assessment of outcome following two-flap palatoplasty in 1184 patients with cleft palate: A retrospective study Highly accessed article p. 84
Sunil Richardson, Shreya Krishna
Aim: The aim of this study is to assess three speech defects, growth impairment, and fistula formation in cleft palate patients after two-flap palatoplasty. Materials and Methods: A total of 1184 patients were included in the study and were followed up for 5–10 years with the minimum age at assessment being 5 years. Speech assessment was done by Perkins perceptual speech assessment. Cast analysis was done to check growth of the patient, and clinically, the presence of fistula was recorded. Wherever needed statistical analysis using SPSS software was done. Results: Speech defects were found in 14.8% of cases. About 75% of these cases were those cases of cleft palate repaired after the age of 2. The rate of fistula formation was 4.3%. Growth impairment as seen by reduced intercanine and intermolar width as well as reduced arch length was seen to be statistically significant. Conclusion: Two-flap palatoplasty is a good technique for cleft palate repair with low rate of speech defects and fistula formation. Growth impairment can be managed by other means. Correct timing of palate surgery is of essence.
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Effectiveness of early intervention on awareness and communication behaviors of mothers of toddlers with repaired cleft lip and palate p. 88
M Pushpavathi, Kavya Vijayan, Akshatha Vishwanath
Objective: The present study is aimed to provide an insight on the causative factors on expressive language delay in toddlers with repaired cleft lip and palate (RCLP). Participants: The present study considered mothers of ten toddlers with RCLP with a mean age of 2.8 years who underwent surgery within 1.5 years. Method / Interventions: The mother-child dyads were enrolled for Early Language Intervention Program (ELIP). The mothers were assessed for the awareness about CLP through a questionnaire and home training was assessed through Mohite's Inventory. Speech and non-speech behaviors of the mothers were assessed using a check list based on an interactional video. These three measures were done before initiating speech and language therapy. Post-therapy measurement was done after 20 sessions. Main Outcome Measures - Pretest post-test design was used to compare the differences in measures for the pre-therapy and post-therapy conditions. Results: Mothers showed greater awareness regarding causes, assessment, treatment, associated problems and issues related to speech therapy in CLP post-orientation (p≤ 0.05). Results also indicated improved home-training abilities of parents on parameters such as language stimulation, physical environment, variety of stimulation and maternal attitude (p≤ 0.05). A statistically significant difference was also noted for speech and non-speech behaviors of the mother (p≤ 0.05). Conclusions: Extensive counseling and orientation helped mothers to gain effective knowledge about cleft lip and palate and focused stimulation approach enabled them to deliver the intervention reliably.
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Evaluation of esthetic outcome following bilateral cleft lip repair using the Mulliken technique: An assessment of 284 cases p. 94
Sunil Richardson, Shreya Krishna
Aim: The aim of the study was to evaluate the esthetic outcome in bilateral cleft lip repairs after using the Mulliken technique using a simplified scale assessed by medical and nonmedical people. Materials and Methods: A total of 284 patients were assessed retrospectively. A three-point scale was devised, and the assessment was carried out on standardized photographs arranged in a presentation format on a screen. The areas assessed were lip, nose, and general facial appearance. Inter-rater reliability was calculated for individual groups and among the groups. Results: The average of the scores of both groups was 2.5 indicating good satisfaction levels. The non medical professionals group gave higher values. The general facial appearance had higher values as compared to the lip and nose assessment. Inter-rater reliability was high. Conclusion: The overall satisfaction levels by both groups were high. Nonmedico group gave higher values, which was considered important as they represent the society that the cleft children interact with. The general facial appearance got high values which shows that individual parts of the face are not scrutinized by individuals. The Mulliken technique of repair gives overall good esthetic results.
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Facial characteristics in Malagasy adults: A pilot study with 64 volunteers p. 98
Nirina Adrien Jean Vivier Mandrano, Yasuyoshi Tosa, Nobuhiro Sato, Tomoaki Kuroki, Shinya Yoshimoto
Context: The facial proportion is essential for preoperative and postoperative assessment in reconstructive and aesthetic surgery. For many years, the neoclassical canon has commonly been used to define the facial morphology features. Aims: The purpose of this study was to assess the validity of neoclassical facial canons and explore its variants in Madagascar population. Settings and Design: This is a descriptive pilot study on facial description of young adult Madagasy volunteers. Subjects and Methods: The study group consists of 64 young adult volunteers, without facial dysmorphy (30 males and 34 females, age 18-30 years). Six neoclassical canons were measured and analyzed. Results were discussed with African, Asian, White Caucasian American and Greek. Results: The three-section facial profile canon was not equal in our population study. The majority of the subjects had a longer forehead and lower face than the middle part; sexual dimorphism was observed. The most frequent variation in the orbital proportion was a wider intercanthal distance. The nose was wider than the intercanthal distance in 100% of the subjects while the mouth width was smaller than the 1.5 nose width. The validity of all canons was poorly demonstrated in our population study. Both African and Asian facial characteristics were observed in our subjects. Conclusion: The majority of our sample does not fit the facial proportion of neoclassical canon. Therefore, it should not be regarded as a directive for facial reconstruction in Malagasy people. These findings can assist surgeons in planning facial plastic surgery for Afro-Asian patients.
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Healthcare providers' psychosocial support for patients with a cleft: Europe and Australia p. 105
Wendy Nicholls, Craig Harper, Serpil Tural, Ieva Maulina, Inta Zepa, Julija Radojicic, Zoran Pešic, Radost Velikova, Evrim Karadag Saygi, Martin Persson
Background: The long-term care of patients with a visible appearance difference may involve many years of multidisciplinary treatment. In addition to providing the physical treatment component, support for the emotional and psychosocial implications must also be considered. The primary objective of this project was to survey healthcare professionals (HCPs) from each of the participating countries to ascertain their level of awareness and understanding of the issues facing those with visible differences, their level of confidence in supporting those patients, and their current and required level of training. Secondary objectives were to gauge the perceived importance of appearance and the psychosocial impact for individuals with visible differences. Methods: Questionnaires were completed by multidisciplinary HCPs in four European countries and Australia. Results: The results for all countries (110 participants) indicate that the majority of HCPs do not feel confident in dealing with the psychosocial needs of people with an appearance disfigurement. Conclusion: All countries identified and supported the need for training of multidisciplinary HCPs to be able to identify and support the psychosocial problems experienced by individuals and families. A collaborative future training program aimed at providing effective psychosocial support and was identified to be the most efficient mode to meet the training requirement. Provision of training will offer the knowledge base for effective psychosocial support and address the unintentional marginalization and social exclusion that may occur by untrained HCPs.
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Cheiloplasty by Pfeifer's Technique p. 113
Rayadurgam Venkata Kishore Kumar, Y Sivanagendra Reddy
Introduction: Cleft lip repair is done usually between the ages of 3rd–5th month of life. Millard's and Tennison techniques are usually used worldwide which are modified Z-plasty techniques at the superior and inferior ends of the philtrum, respectively. Wave-line incision method was introduced by Pfeifer (1970) and the available literature shows that it is an easy technique, applicable to almost all types of clefts. Hence, the study was undertaken to evaluate the results of this method. Patients and Methods: The study was carried out in a total of 701 patients, of which 572 were unilateral cleft lips and 129 were bilateral lips. According to this method, the lip skin incision modified into a wavy line, thus making it less conspicuous using the concept of “morphological order.” The basis of this is that a skin incision between two points can be lengthened if both points are joined in a curve or wavelike manner rather than in a straight line. Results: The results were evaluated for white roll match, vermilion match, cupids bow, and nasal symmetry after cleft repair and found to be excellent with 93.5%, 92.4%, 94%, and 81%, respectively, in unilateral cleft lip variety, good with 75.9%, 71%, 82%, and 87.5%, in bilateral cleft lip variety. Conclusion: This method is not a new technique, but the literature is not available. The technique is found to be good and useful in all clefts, also in revision surgeries and also found to be clinically as well as statistically significant.
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A web based national registry for facial anomalies (DFA) in Bulgaria: An Electronic Medical Record for Facial Anomaly p. 118
Youri Constantinov Anastassov, Kostadin Gigov, Regina Hassan Khater, Radost Velikova Velikova, Maryia Stoyanova Kazakova, Nedyalka Asenova Hashova, Mitko Todorov Bojinov, Petya Svetoslavova Tsarvulanova
Introduction: An original software platform was developed as a partnership between a Parent Organization (www.ALA-BG.org) and a Cleft Center (Medical University Hospital of Plovdiv, Bulgaria). Methods: The software platform enables both the multidisciplinary team and patients to access and update the information referring to certain treatment stages, the treatment plan and the outcome of a stage in a patient's treatment. The information includes data provided by different specialists involved in the multidisciplinary treatment who work at different geographical locations by using the Internet and the original software platform in conformity with the confidentiality and personal data protection requirements. The patient and family are in charge of their own personal data. The patient specifies which specialists can have access and what they may access. All specialists can add their notes and data (text, audio, pictures, video and PDF files) into the patients database. Results: In 2014 the funding for DFA has stopped and ALA receive the rights to develop the database DFA in an Electronic Medical Record, www.emrfa.org. EMRFA was put into operation in 2015, and in 2017 all target functionalities in the system were completed. More than 800 patients are included and the following of the patients have improved substantially. Conclusion: EMRFA can be easily translated into another language and modified for multidisciplinary treatment of other disorders; furthermore, there is a provision for connection and information exchange with other electronic medical records.
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Epidemiologic indices of cleft lip and palate as seen among Igbos in Enugu, Southeastern Nigeria p. 126
Chukwunonso Azubuike Jac-Okereke, Ifeanyi Igwilo Onah
Objectives: The objective of the study was to provide more recent local data, and an estimate of the incidence of cleft lip and/or palate among Igbos. Design: The study design was a descriptive transversal study of birth records and case notes between January 2007 and December 2011 and records of clinical attendance and interviews with patients' mothers. Names and state of origin were used as inclusion criteria for Igbos. Setting: Previous local data on epidemiology are over three decades old. The National Orthopaedic Hospital, Enugu (NOHE) is an apex center for plastic surgery in southeastern Nigeria. Enugu State University of Science and Technology Teaching Hospital (ESUTTH) is the largest state-owned health facility. There are ethnoracial variations in the epidemiology of clefts. Materials and Methods: All patients born with clefts at ESUTTH and all records of clefts in the period available at NOHE hospital in the period. Within the period, 262 cleft patients were studied: 139 males and 123 females; male:female 1.13:1. Main Outcome Measure: Main outcome measures were the live birth incidence of clefts, the most frequently occurring birth order, and socioeconomic class affected. Results: There were 5810 live births with a 0.00103270223752151 birth incidence. That among Igbos was 0.0010700909577314. Infants in the first birth order and of low socioeconomic class accounted for the highest incidence. The socioeconomic status was found to be statistically significant (P = 0.0001, confidence interval: 95%) but birth order was not (P = 0.932). There was a positive family history in 9.5% with first-degree relatives accounting for 6%. The mean maternal age was 28.88 years (range: 17–47 years), and mean paternal age was 38.55 years (range: 23–58 years). Conclusion: The epidemiologic indices differ from data published over three decades ago. There are differences in cleft epidemiology within Nigeria.
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Total immersion speech camps for patients with cleft palate p. 132
María Del Carmen Pamplona, Pablo Antonio Ysunza
Background: Children with cleft palate (CCP) frequently shows language and/or speech disorders. However, a significant number of children cannot receive speech pathology treatment on a regular basis. For these children, new modalities of intervention have to be developed for stimulating speech and language. Objective: The purpose of this paper is to study and compare the two modalities of speech intervention in CCP: a conventional approach providing speech therapy in 1 h sessions, once a week, and a total immersion speech camp in which CCP received therapy within a naturalistic environment 10 h/day for a period of 5 days. Materials and Methods: Twenty-three children were studied. A matched control group was assembled. Children included in the experimental group attended a total immersion speech camp for 5 days. Matched control children received speech therapy once per week in 1 h sessions. Results: When comparing the advances of the CCP participating in total immersion speech camps to CCP assisting to speech therapy 1 day a week, significant similar advances (P > 0.05) in articulation were demonstrated. Moreover, significant improvements (P < 0.05) were found when articulation deficits were compared before and after the speech treatment periods. Conclusion: Total immersion speech camps seem to be a valid and reliable option for speech intervention in CCP.
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Comparison of soft-tissue changes after the surgical repair of unilateral cleft lip side to noncleft lip side: An anthropometric study p. 139
Mohamed Abdul Wajid, Rohit Kulshrestha, Ramji Rastogi, Deepak Kumar, Kamlesh Singh, Mohamed Abdul Ateeq
Aim: The aim of this article was to compare the tissue contraction after the surgical repair of unilateral cleft lip side to noncleft lip side. Materials and Methods: Forty patients with unilateral cleft lip only were used as subjects. They were divided into two groups based on gender (20 males and 20 females). Measurements of soft-tissue landmarks of cleft side and noncleft side were compared after surgical repair of the lip to check for soft-tissue contractions. A vernier caliper was used to measure the linear measurements, and all the measurements were measured directly on the patients' lips. Results: When gender comparison was done, it was found to be significant only for oral commissure to the peak of Cupid's bow for both cleft and noncleft sides (P < 0.05). When comparison between the two sides (left and right) was done, a significant difference was observed only for subalar to peak of Cupid's bow measurement which was found to be significantly larger at noncleft as compared to cleft side in overall assessment (P = 0.005) and in females (P = 0.046); however, this difference was not statistically significant when evaluated for males alone (P = 0.060). Conclusion: Marked soft-tissue contraction was seen after surgical repair of the unilateral cleft lip, more was seen in females. This indicates that adequate planning and accurate surgical techniques and measurements should be taken while approximating the two lip flaps both in vertical and in horizontal direction before planning the surgery to get the best esthetic result for the patients.
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Direct Anthropological analysis of Unilateral Cleft Nose: A Novel Approach using an Innovative Plane p. 144
Sanchit Jain, Gunjan Dube
Objective: The main objective of this study is to introduce a new plane for direct anthropological study of the nose. Design: The study is a cross-sectional study done to analyze cleft nasal deformity by direct method and comparing with indirect method for assessing reliability of the plane introduced. Setting: The study was carried out in institutional setup which is approved for smile train cleft project. Inclusion Criteria: The study included only those patients who were affected with unilateral cleft lip isolated or associated with cleft palate. All the patients who were included in the study were aged above 15 years where the growth of the nose was completed. Exclusion Criteria: Patients who are aged below 15 years of age, whose growth is yet not complete. Main Outcome Measure(s): There had been no specific plane to analyze the angle of columella in recent past which has been mostly analyzed by indirect methods (photographic method and three-dimensional scans). We have attempted to introduce a new plane for analysis of nose anthropologically both using direct method and compared it with the indirect method for its reliability. Results: Comparative assessment with indirect method shows a little variation in recording which is not statistically significant, thus ensuring the reliable application of this particular technique for evaluating cleft nasal deformity. Conclusions: The technique described is easier to apply and replicate in limited resource setup as it does not require high-end armamentarium and equipment.
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Oral health status among cleft lip and palate patients in South India: A profile p. 152
Chitta Ranjan Chowdhury, Shahnawaz Khijmatgar, Nanda P Kishore, Vikram Shetty
Background: Cleft lip and palate (CLP) is one of the identified anomalies in India. Many of the CLP cases have compromised oral health status which relates to their quality of life (QoL). Therefore, it is a need to assess their oral health status in terms of dental caries, periodontal disease, hypodontia, malocclusions, etc. Hence, the objective of this study is to investigate the oral health status among CLP cases. Material and Methods: A questionnaire survey was designed for a total of 300 CLP cases that came to the Nitte Meenakshi Institute of Craniofacial Surgery of Nitte University, India, were included in the study. A questionnaire was designed and pretested, and informed consent from the cases and carers was taken. Results: The average mean age of the patients with CLP was 17.48 (3–47). There were 47% males (n = 141) and 53% females (n = 159). 63% (n = 170) of them came from low socioeconomic background. Out of 300 cases, 31.0% (n = 90) had cleft lip; 1.0% (n = 3) had cleft palate, and 67.8% (n = 196) of them had both CLP. Most of the cases, i.e., 65.4% (n = 196) were unilateral CLP and 52.7% (n = 158) had right-sided clefts. The literacy status of the parents, their socioeconomic status, and occupation had a significant interrelation for the occurrence of CLP (P < 0.05). Similarly, diminutive or peg-shaped tooth, hypodontia, facial profile, and absence of lateral incisors in the line of cleft showed a significant association of this CLP condition. There was also strong interrelation between the presence of postoperative intraoral scarring in patients affected with the CLP (P < 0.05). The score for decayed missing and filled teeth (DMFT/dmft) was 5.16 for 6–12 years old and >9 in >13 years old, community periodontal index was between 0 and 2, and the simplified oral hygiene index was 0.9–1.41. There were cases of AQ3 hypodontia, hypomineralization, and malocclusion. The statistical test was performed using ANOVA. Conclusion: We conclude that, there was a moderate dental caries risk. The periodontal status was fair. There was association of occurrence of hypodontia and malocclusions in craniofacial anomalies. This affects their QoL.
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The relationship between cleft palate index and oronasal fistula p. 160
Ezekwe Ekwueme Amirize, Ifeanyichukwu Igwilo Onah, Tombari Joseph Gbeneol, Oladimeji Akadiri Adeniyi
Aims and Objectives: To identify fistula rates and determine the relationship between cleft palate index (CPI) and oronasal fistula. To delineate palatal index with high risk of fistulation in our study population. Materials and Methods: A 1-year prospective study. All consecutive patients with cleft palate that presented to the study institutions within the period and met inclusion criteria were studied. Palatal cleft width was measured in millimeters using fine pointed calipers at the level of the tuberosities intra-operatively. Maxillary intertuberosity width was measured. CPI was calculated as a ratio of cleft width to maxillary width. Palatoplasty was by von Langenbeck repair or intravelar veloplasty. Data were documented and analyzed. P < 0.05 were considered statistically significant. Results: Forty-six patients were studied. Ages ranged from 10 months to 28 years. Ranges of measured parameters were cleft width 4–25 mm, maxillary width 35–82 mm and CPI 0.07–0.49. Nineteen patients developed oronasal fistula; 41.30% overall fistula rate. Likelihood of fistula occurring increased as palatal index increased. Fistula was common in patients with CPI in excess of 0.22. Risk of fistula formation was statistically significant, becoming higher with palatal index of 0.26 and above. Discussion and Conclusion: Cleft palate exhibits great variability in tissue deficiency and segments separation. This may influence cleft severity. Palatal index estimates cleft severity and allows identification of patients at risk of fistula formation thereby necessitating individualized surgical attention for a better outcome.
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Evaluation of dental arch relationship of patients with bilateral cleft lip and palate applying bilateral yardstick p. 167
Cristiane Lucas de Farias Luz, Terumi Okada Ozawa, Rafael Arouca, Amanda Ohashi, Daiana Broll
Aims: This study aims to verify the applicability and reliability of the Bilateral yardstick on the assessment of interarch relationship in patients with bilateral cleft lip and palate (BCLP) through three-dimensional (3D) virtual casts. Setting and Design: A total of 112 individuals with BCLP, aging 6–12 years old, with no associated syndromes, free from previous orthodontic treatment and from secondary bone grafting. Methods: Plaster casts from the 112 individuals were obtained, randomly numbered and scanned, generating a 3D digital image. Three calibrated orthodontists used the Bilateral yardstick to classify, in two rounds, the interarch relationship on the virtual casts. Descriptive statistics were employed to describe the frequencies of the scores of the Bilateral yardstick. Weighted Kappa statistics were calculated to determine intra- and inter-rater reliability. Results: Raters reported no difficulties in the employment of the Bilateral yardstick on 3D virtual casts. Good occlusal relationship or minor deviation was observed in 60 (53.6%) individuals. No more than 13 (11.6%) patients were classified as having a poor arch form. Intraexaminer kappa values were higher than 0.93 (confidence interval [CI] 0.88–0.98), and interexaminer agreement was higher than 0.78 (CI 0.69–0.87). Conclusion: Bilateral yardstick proved to be applicable to the assessment of dental arch relationship of patients with BCLP through 3D virtual casts. Weighted kappa values indicated good intrarater and good-to-very good agreement, supporting the reliability of this yardstick when employed to virtual casts. Further studies should be encouraged to face the actual lack of evidence in this field.
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The evaluation of the effects of rapid palatal expansion on nasomaxillary complex in patients with complete unilateral and bilateral cleft lip and palate p. 173
Ege Dogan, Servet Dogan
Aim: The aim is to evaluate the effects of rapid palatal expansion (RPE) in children with complete unilateral and bilateral cleft lip and palate (UCCLP/BCCLP), compared with noncleft patients. Materials and Methods: This study includes 45 patients aged between 8 and 11 years old (15 patients with UCCLP; mean age: 9 years 2 months, 15 patients with BC-CLP; mean age: 9 years, 15 patients with noncleft; mean age: 9 years 1 month), in Ege University, Department of Orthodontics. RPE was applied before secondary alveolar grafting. Posteroanterior cephalograms were taken before and after expansion. One-way analysis of variance was used to analyze the differences among the groups, whereas the paired t-test was used to evaluate the changes in each group. Results: In all groups, RPE causes a significant increase of the internasal dimension and maxillary width (Ln-Ln', Mx-Mx'). In the cleft groups, especially in the bilateral CLP group, the nasal width (Ln-Ln') and maxillary width (Mx-Mx') show a statistically significant increase according to the control group (P < 0.001). Furthermore, the unilateral CLP group shows an increase according to the control group (P < 0.001).(Um-Um') and (Lm-Lm') show a statistically significant increase all in three groups compared before the treatment (P < 0.001).(Um-Um') show the most increase in bilateral CLP group (P < 0.001). In the bilateral CLP group, (Zyg-Zyg'), (Cdl-Cdl'), (Go-Go') show a statistically significant increase (P < 0.001). The control and unilateral CLP group also show an increase, but the unilateral CLP group is not found statistically significant. Conclusion: When the facial morphologies were evaluated after RPE, the facial width measurements of the bilateral CLP patients showed a greater increase than the unilateral CLP and control group. Nasal width measurements were wider in cleft groups compared to the control group contributing to a widened airway, which may have an effect on breathing and speaking in cleft patients. More studies are needed.
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Three methods comparison using two-dimensional software (a novel technique), tri-dimensional cone-beam computed tomography, and manual method to measure maxillary casts: Unilateral and bilateral cleft lip and palate infants up to 6 months p. 180
Faizan Ahmed Khan, Mohammadi Begum Khan, Akhter Hussain, Arjun Karra, Purumandla Usha, CH Lalitha
Objective: The objective of the study was to investigate any relationship between three different methods of measurements used to measure certain parameters used on the casts of infants born with cleft lip and palate (CLP). Materials and Methods: A set of 25 casts including both unilateral and bilateral CLP were used to determine the relationship among three different methods used in this study, including tri-dimensional (3D) cone-beam computed tomography, two-dimensional (2D) software, and manual method using Vernier caliper. Results: Linear regression analysis or regression curves were attempted to establish a relationship among three different methods. Statistically significant difference (P < 5%) was found for almost all the measurements analyzed. The measurements obtained by Vernier caliper were found to be close to software values than 3D values. The ability of obtaining a 3D value from a measured 2D value was determined as the linear Pearson's correlation coefficient R and the amount of scatter around the regression line as represented by 95% confidence interval. Total error of 3D value calculated from 2D measurements was obtained by regression analysis. Conclusion: Through this study, it was concluded that newer and simpler methods for measurement purpose are always accepted on a wider scale and can be employed universally. We could find that 2D measurements by Vernier caliper which is closer to software measurements (using MAKHTER Software) can be converted to 3D using a multiplication factor of 0.54.
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Three methods comparison using two-dimensional software (a novel technique), three-dimensional-cone-beam computed tomography, and manual method to measure maxillary casts: Unilateral and bilateral cleft lip and palate infants up to 6 months p. 187
N Madhumitha
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Our unified pharyngeal flap operation p. 189
Mikihiko Kogo, Takayoshi Sakai, Takeshi Harada, Kanji Nohara, Emiko Tanaka Isomura, Tetsuya Seikai, Koichi Otsuki, Chihiro Sugiyama, Kiyoko Nakagawa, Susumu Tanaka
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Double-angled palatal needle holder: An innovative instrument for cleft surgeries p. 192
BS Bibilash, S Dinesh Kumar, Ravi Kumar Chittoria, Devi Prasad Mohapatra, MT Friji, KS Krishnakumar
Introduction: The devices used for cleft surgeries are different from the routine plastic surgical ones. The currently available needle holders for palatal surgeries do not address many issues like poor visibility and excessive strain for the surgeon's dominant hand. We present an innovative needle holder which could alleviate these problems. Materials and Methods: The new needle holder was inspired from the design of a Magill forceps. It was modified to a needle holder and later on the angles were adjusted for using in palatoplasty surgery. Results: The final device had following advantages: Surgeon's hands are outside the target visual field and no blind spots or shadows in the field, surgeon's wrists, elbows and shoulders were in a more comfortable position while operating from the head end. Conclusion: The double angle needle holder was really useful for oral surgeries especially palatoplasty. The position of the surgeon's hands was more comfortable; they had better visibility and was easier to maneuver.
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Presurgical nasoalveolar molding therapy in patients with bilateral cleft lip and palate Highly accessed article p. 195
Lourdes Martínez Motta, Jessica Sánchez Huanca
Infants with bilateral cleft lip and palate have an esthetic and functional involvement of the face. To obtain esthetic results, such as reducing the cleft gap and mediating premaxilla, various treatments are performed, such as presurgical orthopedics with extraoral traction headgear and nasoalveolar molding. Nasoalveolar molding (NAM) therapy has proven to be an effective method for reducing the cleft, improving the anatomy of the nose and remodeling the alveolar and nasal segments before surgical repair. This article presents two clinical cases with bilateral cleft lip and palate; case 1 treated with presurgical orthopedics with extraoral traction headgear and case 2 treated with NAM therapy.
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Median cleft lip: A case series from a rural cleft center p. 198
NR Salampuria, Mayur B Agrawal, Anmol Chugh
Median cleft lip is a midline craniofacial cleft through upper lip. The incidence of craniofacial clefts is about 1:1,000,000 of live births. It indicates the rarity of this anomaly. Hence, this series is being presented. From January 2010 to December 2017, eight cases of the median cleft of upper lip attended the cleft clinic based in rural India. All eight patients had partial midline cleft lip. One patient had abnormal soft tissue mass and one patient had hypertelorism with nasal deformity. All of them underwent surgical repair of median cleft lip using inverted-U incision along with repair of orbicularis oris muscle in the midline. The postoperative symmetry was achieved in 100% of patients with good reconstruction of the philtrum. The associated deformities were left to be managed in second stage. A series of eight cases of partial midline cleft lip has been presented with good surgical outcome.
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Congenital hemifacial hyperplasia with “locked jaw”: Rare anomaly with a rare presentation p. 201
Parvathi Ravula, Srikanth Rangachari, K Swathi
Congenital hemifacial hypertrophy is a rare developmental disorder characterized by unilateral enlargement of all the facial tissues of viscerocranium involving the teeth, bone, and soft tissues. Many case reports have been reported in the literature describing the characteristic clinical and radiological features, etiology, and conditions mimicking the hemifacial hypertrophy. In general, treatment is considered for the esthetic improvement in the form of soft tissue debulking, osteotomies, or orthognathic procedures. We report a case of true hemifacial hypertrophy presented with a severe temporomandibular joint ankylosis with the jaws locked in an anterior open bite deformity and its management.
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Oculocardiac reflex: An incident reporting during metopic craniosynostosis repair p. 205
Deepak Dwivedi, Ashish Chauhan, Arijit Ray, Subramanya GS Datta
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